MANAGEMENT OF REFRACTORY LYMPHATIC LEAK
CASE 2: A newborn male is admitted to the neonatal ICU for respiratory distress secondary to large bilateral pulmonary effusions. The infant is intubated and multiple chest tubes are placed to maintain adequate ventilation. Aspirated fluid is milky white in color and confirmed chylous with a high triglyceride and lymphocyte content. Peripheral laboratory evaluation is notable for hypoalbuminemia, hypogammaglobulinemia and lymphocytopenia. In addition to continuous chest tube drainage, acute interventions include complete gut rest with total parenteral nutrition and intravenous lipids, IV albumin replacement, and initiation of octreotide infusion. Despite maximizing these interventions, chylous output remains > 20ml/kg/day. Intranodal Dynamic Contrast-Enhanced MR lymphangiography (IN-DCMRL) demonstrates a diffuse Central Conducting Lymphatic Anomaly (CCLA) with extensive abdominal and thoracic lymphangiomatosis. Interventional radiology acutely performs lymphatic interstitial embolization while hematology/oncology initiates oral sirolimus pharmacotherapy. After 8 weeks of sirolimus therapy, although now extubated and protein replete, the infant remains with a single chest tube in place and continues to struggle advancing beyond a medium chain triglyceride formula.
Surgical and endolymphatic strategies for treatment of congenital chylous effusions and ascites are usually employed after failure of medical therapies. The decision to perform an intervention depends on factors related to local expertise. Many times, these invasive procedures are performed in tandem or after prior procedures have failed to stop chylous leakage.
Symptoms of refractory disease include unresponsive daily loss of chyle exceeding 100 ml/day for a 5-day period, or symptomatic nutritional, protein and/or electrolyte complications. Infants should not be allowed to progress to a state of severe hepatic cholestasis without an attempt at intervention.[21] Surgical and endolymphatic interventions include drainage, shunting, pleurodesis, ligation of lymphatics, lymphovenous anastomosis, and thoracic duct embolization.[22]